Heart Syndrome

A syndrome refers to the association of several recognizable features, such as signs and symptoms, which GeneRally occur together, allowing one feature to alert a physician to the presence of others. Heart syndromes occur when an individual experiences signs or symptoms of a particular heart disorder. These signs and symptoms alert the physician, allowing them to diagnose the patient and administer treatment.

Types of heart syndromes

• Hypoplastic left heart syndrome (HLHS)
• Hypoplastic right heart syndrome (HRHS)

Hypoplastic left heart syndrome and hypoplastic right heart syndrome are heart disease syndromes that are congenital conditions, meaning that the conditions were present in the individual at birth, as opposed to developing over the course of the individual's life. The left or right structure of the heart is underdeveloped, which in turn causes newborn babies to present a bluish coloration that can be observed in the chest, abdomen, lips, and tongue. When underdevelopment occurs within a given structure of the heart, blood will flow into the coronary arteries abnormally, in turn causing damage to the heart muscles.

When this lack of development occurs within the right structure of the heart (HRHS), the absence of vital organ parts and functions may occur, such as the absence of the pulmonary asteria valve, the valve which opens and closes, allowing blood flow to the lungs.

When this lack of development occurs within the left structure of the heart (HLHS), the aorta, left ventricle aortic valve and mitral valve May Be subjected to underdevelopment.

Causes

Congenital cardiovascular defects are present in AbOUT 1% of live births, and are the most common Congenital malformations in newborns. Unfortunately, heart syndromes tend to occur sporadically, without any particular reason. For one reason or another, a region of the heart remains underdeveloped, leaving certain parts of the heart inoperable. There are no steps of precaution that an individual can take to avoid heart syndromes, as said defects occur while the fetus is still developing within its mother. Heredity may also play a role in the formation of congenital heart defects, as well as the mother's consumption of alcohol and drugs during the nine months of pregnancy.

Symptoms

The most common symptom of heart syndrome is chest pain, also known as angina, and can be described as a discomfort, heaviness, pressure, aching, burning, fullness, squeezing, or painful feeling within an individual's chest. Parents and physicians should immediately take note if an individual (especially a newborn) is suffering from the following symptoms:

• Difficulty sucking and/or feeding
• Lethargy
• Rapid breathing and/or shortness of breath
• Cold extremities
• Enlarged liver
• Poor pulse
• Pounding heart
• Bluish (cyanosis) or poor skin color

Treatment

Once a newborn has been diagnosed with a heart syndrome, it will then be admitted into the neonatal intensive care unit. If needed, a ventilator may be placed on the baby to help it breathe. The baby will also be administered a medicine called prostaglandin E1, which will maintain circulation of blood throughout the baby's body.

Unfortunately, these measures do not solve the congenital heart problems, as this condition always requires surgery. The first surgery, known as the Norwood operation, occurs within the first few days of the newborn's life, and consists of the building or a new aorta. An artificial shunt is also inserted to maintain blood flow to and from the lungs. If everything goes as planned, the newborn is usually transferred home a day or two post surgery. The newborn will need to be administered one or more daily medications and be closely followed by a pediatric cardiologist, who will then make the decision of when the newborn baby should undergo the second stage of surgery. The second portion of the operation is called the Glenn shunt, or Hemifontan procedure. During this procedure, half of the veins carrying blue blood from the body are connected directly to blood vessels to the lungs, allowing for oxygen to be received. This procedure is usually performed when the child is four to six months of age. During the first two surgical procedures, the child may still appear somewhat blue. The final portion of the operation is called the Fontan procedure. During this procedure, the remaining veins carrying blue blood from the body are connected directly to the blood vessels to the lungs, making the right ventricle the only pumping chamber of the heart. This procedure is usually performed when the child is between the ages of eighteen months and three years of age. Once the final portion of the operation is completed, the baby will no longer appear blue. It isn't uncommon for some patients to require further surgeries in their 20's or 30's if they develop arrhythmias, or other complications that occur due to the Fontan procedure. Some hospitals and medical professionals feel that a heart transplant is a better choice than the three steep surgery process. Unfortunately, however, there are very few donated hearts available for infants.

Complications

Complications may include:

• Irregular, fast heart rhythms, which is known as arrhythmias
• Chronic diarrhea caused by a disease called protein loosing enteropathy.
• Fluid within the abdomen (ascites) and in the lungs (pleural effusion)
• Blockage of the artificial shunt
• Strokes and other neurological complications
• Sudden death